Idiopathic Hyperammonemia after Orthotopic Lung Transplantation - Juniper Publishers - Journal of Anesthesia & Intensive Care Medicine

Juniper Publishers - Open Access Journal of Journal of Anesthesia & Intensive Care Medicine

Idiopathic Hyperammonemia after Orthotopic Lung Transplantation

Authored by Tyson Kathleen

Idiopathic hyperammonemia is characterized by increased serum ammonia levels (>200umol/L) and is sometimes associated with normal to slightly elevated liver function tests [1]. These patients often present with encephalopathy, cerebral edema, seizures, and coma. Idiopathic hyperammonemia has been reported after high dose chemotherapy, and organ transplants including Orthotopic lung transplant [2]. Hyperammonemia is a rare, severe, and often fatal complication. There are few cases reported in the literature since 1997, including one retrospective analysis of 807 lung transplants; only 8 patients were diagnosed with hyperammonemia [3]. The exact mechanism of idiopathic hyperammonemiais not fully understood with congenital or acquired defects in ammonia metabolism, as well as bacterial infection having been described [3].
Ammonia is primarily cleared by the liver’s urea cycle. The urea cycle converts ammonia to urea through a series of intermediate reactions. Hyperammonemia can be the result of any number of inborn errors (congenital or acquired) of urea cycle metabolism. Another hepatic mechanism for ammonia clearance is through glutamate synthetase, which converts glutamate and ammonia to glutamine. In the kidneys, glutamine is broken down back into glutamate and ammonia, which is then excreted. If hepatic or renal elimination of ammonia is interrupted, then hyperammonemia can occur leading to severe consequences. We present a case below of a female patient who developed hyperammonemia after Orthotopic lung transplant.


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